TY - JOUR
T1 - Consensus statement on the pathology of IgG4-related disease
AU - Deshpande, Vikram
AU - Zen, Yoh
AU - Chan, John K.C.
AU - Yi, Eunhee E.
AU - Sato, Yasuharu
AU - Yoshino, Tadashi
AU - Klöppel, Günter
AU - Godfrey Heathcote, J.
AU - Khosroshahi, Arezou
AU - Ferry, Judith A.
AU - Aalberse, Rob C.
AU - Bloch, Donald B.
AU - Brugge, William R.
AU - Bateman, Adrian C.
AU - Carruthers, Mollie N.
AU - Chari, Suresh T.
AU - Cheuk, Wah
AU - Cornell, Lynn D.
AU - Fernandez-Del Castillo, Carlos
AU - Forcione, David G.
AU - Hamilos, Daniel L.
AU - Kamisawa, Terumi
AU - Kasashima, Satomi
AU - Kawa, Shigeyuki
AU - Kawano, Mitsuhiro
AU - Lauwers, Gregory Y.
AU - Masaki, Yasufumi
AU - Nakanuma, Yasuni
AU - Notohara, Kenji
AU - Okazaki, Kazuichi
AU - Ryu, Ji Kon
AU - Saeki, Takako
AU - Sahani, Dushyant V.
AU - Smyrk, Thomas C.
AU - Stone, James R.
AU - Takahira, Masayuki
AU - Webster, George J.
AU - Yamamoto, Motohisa
AU - Zamboni, Giuseppe
AU - Umehara, Hisanori
AU - Stone, John H.
PY - 2012/9
Y1 - 2012/9
N2 - IgG4-related disease is a newly recognized fibro-inflammatory condition characterized by several features: a tendency to form tumefactive lesions in multiple sites; a characteristic histopathological appearance; andoften but not alwayselevated serum IgG4 concentrations. An international symposium on IgG4-related disease was held in Boston, MA, on 4-7 October 2011. The organizing committee comprising 35 IgG4-related disease experts from Japan, Korea, Hong Kong, the United Kingdom, Germany, Italy, Holland, Canada, and the United States, including the clinicians, pathologists, radiologists, and basic scientists. This group represents broad subspecialty expertise in pathology, rheumatology, gastroenterology, allergy, immunology, nephrology, pulmonary medicine, oncology, ophthalmology, and surgery. The histopathology of IgG4-related disease was a specific focus of the international symposium. The primary purpose of this statement is to provide practicing pathologists with a set of guidelines for the diagnosis of IgG4-related disease. The diagnosis of IgG4-related disease rests on the combined presence of the characteristic histopathological appearance and increased numbers of IgG4 plasma cells. The critical histopathological features are a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. We propose a terminology scheme for the diagnosis of IgG4-related disease that is based primarily on the morphological appearance on biopsy. Tissue IgG4 counts and IgG4:IgG ratios are secondary in importance. The guidelines proposed in this statement do not supplant careful clinicopathological correlation and sound clinical judgment. As the spectrum of this disease continues to expand, we advocate the use of strict criteria for accepting newly proposed entities or sites as components of the IgG4-related disease spectrum.
AB - IgG4-related disease is a newly recognized fibro-inflammatory condition characterized by several features: a tendency to form tumefactive lesions in multiple sites; a characteristic histopathological appearance; andoften but not alwayselevated serum IgG4 concentrations. An international symposium on IgG4-related disease was held in Boston, MA, on 4-7 October 2011. The organizing committee comprising 35 IgG4-related disease experts from Japan, Korea, Hong Kong, the United Kingdom, Germany, Italy, Holland, Canada, and the United States, including the clinicians, pathologists, radiologists, and basic scientists. This group represents broad subspecialty expertise in pathology, rheumatology, gastroenterology, allergy, immunology, nephrology, pulmonary medicine, oncology, ophthalmology, and surgery. The histopathology of IgG4-related disease was a specific focus of the international symposium. The primary purpose of this statement is to provide practicing pathologists with a set of guidelines for the diagnosis of IgG4-related disease. The diagnosis of IgG4-related disease rests on the combined presence of the characteristic histopathological appearance and increased numbers of IgG4 plasma cells. The critical histopathological features are a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. We propose a terminology scheme for the diagnosis of IgG4-related disease that is based primarily on the morphological appearance on biopsy. Tissue IgG4 counts and IgG4:IgG ratios are secondary in importance. The guidelines proposed in this statement do not supplant careful clinicopathological correlation and sound clinical judgment. As the spectrum of this disease continues to expand, we advocate the use of strict criteria for accepting newly proposed entities or sites as components of the IgG4-related disease spectrum.
KW - IgG4
KW - IgG4-related disease
KW - consensus statement
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UR - http://www.scopus.com/inward/citedby.url?scp=84865767615&partnerID=8YFLogxK
U2 - 10.1038/modpathol.2012.72
DO - 10.1038/modpathol.2012.72
M3 - Article
C2 - 22596100
AN - SCOPUS:84865767615
SN - 0893-3952
VL - 25
SP - 1181
EP - 1192
JO - Modern Pathology
JF - Modern Pathology
IS - 9
ER -